Clinical–Pathological Conference Rare Cause of Severe Hypertension in a Young Woman Colin G. Perry, E. Marie Freel, Patrick O’Dwyer, Ernesto L. Schiffrin, Garry L. Jennings, Anna F. Dominiczak, Marc De Buyzere Presentation of Case
Discussion of Investigation
An asymptomatic 20-year-old woman was seen at a preoperative assessment clinic before elective orthopedic surgery. She had a blood pressure of 240/110 mm Hg in both arms and was referred for urgent medical opinion. On examination, her phenotype was unremarkable. She had bilateral papilledema with normal cardiovascular examination other than her blood pressure. Renal function and urinalysis were normal. The patient had a normal renal ultrasound, plain computed tomography of brain, and computed tomography cerebral venogram. Renin and aldosterone were normal with a normal ratio and potassium was within the normal range. She commenced amlodipine and an angiotensin-converting enzyme inhibitor with blood pressure falling to 151/112 mm Hg. Dr Colin Perry: I would ask our experts, what would they do next? Dr Ernesto Schiffrin: I think in a young person you have to consider secondary hypertension. And of course, with very severe elevated blood pressure I would have liked to know whether she is very pale, does she not have headaches? One would start to think of pheochromocytoma in a young person like this. Dr Perry: That is why I think the history is very important at this stage, because she did not complain of recent headaches when seen initially in the emergency department. She was always rather pale. Dr Schiffrin: I think that is important because I always tell the medical students that if the patient has a flushed face it is not pheochromocytoma. Pallor is almost an inevitable element. Dr Marie Freel: Would there have been an argument that an MR angiogram of renal vessels might still have been appropriate in a young girl with malignant-phase hypertension because you can still have a fairly normal aldosterone renin ratio in that circumstance? Dr Perry: I think that is entirely justifiable.
Dr Perry: Can I ask then, what test would you do for pheochromocytoma? Dr Schiffrin: We usually measure urinary metanephrines. Prof Garry Jennings: This lady did not have it, but when you have episodic hypertension with a pheochromocytoma, that is when your plasma measurement can be absolutely definitive. If they are not elevated and their blood pressure is up, they have not got a pheochromocytoma. Dr. Schiffrin: Just going back to the history, simply because I have seen some of these patients, I would have inquired about family history, about thyroid surgery in the family. Dr. Perry: I agree, however at this stage, she has not been to the specialist center. So, in fact you are correct. She does have headaches and if you really explore the history, she gives a good story of headaches. Eighty percent of patients with pheochromocytoma will give a history of headaches when asked specifically. Twenty-four–hour urinary metanephrines and catecholamines were measured and were reported as elevated, with 24-hour urinary norepinephrine 11 374 nmol/24 hour (normal range
Discussion of Investigation
An asymptomatic 20-year-old woman was seen at a preoperative assessment clinic before elective orthopedic surgery. She had a blood pressure of 240/110 mm Hg in both arms and was referred for urgent medical opinion. On examination, her phenotype was unremarkable. She had bilateral papilledema with normal cardiovascular examination other than her blood pressure. Renal function and urinalysis were normal. The patient had a normal renal ultrasound, plain computed tomography of brain, and computed tomography cerebral venogram. Renin and aldosterone were normal with a normal ratio and potassium was within the normal range. She commenced amlodipine and an angiotensin-converting enzyme inhibitor with blood pressure falling to 151/112 mm Hg. Dr Colin Perry: I would ask our experts, what would they do next? Dr Ernesto Schiffrin: I think in a young person you have to consider secondary hypertension. And of course, with very severe elevated blood pressure I would have liked to know whether she is very pale, does she not have headaches? One would start to think of pheochromocytoma in a young person like this. Dr Perry: That is why I think the history is very important at this stage, because she did not complain of recent headaches when seen initially in the emergency department. She was always rather pale. Dr Schiffrin: I think that is important because I always tell the medical students that if the patient has a flushed face it is not pheochromocytoma. Pallor is almost an inevitable element. Dr Marie Freel: Would there have been an argument that an MR angiogram of renal vessels might still have been appropriate in a young girl with malignant-phase hypertension because you can still have a fairly normal aldosterone renin ratio in that circumstance? Dr Perry: I think that is entirely justifiable.
Dr Perry: Can I ask then, what test would you do for pheochromocytoma? Dr Schiffrin: We usually measure urinary metanephrines. Prof Garry Jennings: This lady did not have it, but when you have episodic hypertension with a pheochromocytoma, that is when your plasma measurement can be absolutely definitive. If they are not elevated and their blood pressure is up, they have not got a pheochromocytoma. Dr. Schiffrin: Just going back to the history, simply because I have seen some of these patients, I would have inquired about family history, about thyroid surgery in the family. Dr. Perry: I agree, however at this stage, she has not been to the specialist center. So, in fact you are correct. She does have headaches and if you really explore the history, she gives a good story of headaches. Eighty percent of patients with pheochromocytoma will give a history of headaches when asked specifically. Twenty-four–hour urinary metanephrines and catecholamines were measured and were reported as elevated, with 24-hour urinary norepinephrine 11 374 nmol/24 hour (normal range
